PGC-1α, mitochondrial dysfunction, and Huntington's disease

Ashu Johri, Abhishek Chandra, M. Flint Beal
2013 Free Radical Biology & Medicine  
The constant high energy demand of neurons makes them rely heavily on their mitochondria. Dysfunction of mitochondrial energy metabolism leads to reduced ATP production, impaired calcium buffering, and generation of reactive oxygen species. There is strong evidence that mitochondrial dysfunction results in neurodegeneration and may contribute to the pathogenesis of Huntington's disease (HD). Studies over the past few years have implicated an impaired function of peroxisome
more » ... receptor (PPAR)-γ coactivator-1α (PGC-1α), a transcriptional master co-regulator of mitochondrial biogenesis, metabolism and antioxidant defenses, in causing mitochondrial dysfunction in HD. Here we have attempted to discuss in a nutshell, the key findings on the role of PGC-1α in mitochondrial dysfunction in HD and its potential as a therapeutic target to cure HD.
doi:10.1016/j.freeradbiomed.2013.04.016 pmid:23602910 pmcid:PMC3722269 fatcat:ewnmj5fc45glbauh2h35c2ep5u